11 results
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
IgG4-Related Disease ... Clinical history ... concentration PET-CT ... #diagnosis #management ... #rheumatology
VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) Syndrome
Clinical Syndrome:
 • Common Clinical Features: alveolitis, ear and
Clinical Syndrome ... thromboembolic disease ... • giant cell arteritis ... myeloid cells NEJM ... Autoinflammatory #Somatic #rheumatology
Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young
association • M > F Clinical ... (Clinical Dx). ... , AS Treatment: ... #diagnosis #management ... signs #symptoms #rheumatology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
Clinical Presentation ... rash) • Heart, lung ... Treatment: • Corticosteroids ... #Diagnosis #Management ... #Hematology #Rheumatology
Giant cell arteritis (GCA)

Giant cell arteritis (GCA) definition: Most common systemic inflammatory vasculitis in older adults
Diagnosis = clinical ... in literature (NEJM ... cases (Cornelia NEJM ... them, but urgent rheumatology ... Symptoms #Diagnosis #Management
Common Variable Immunodeficiency (CVID)

What?
CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
to 8 years Clinical ... • Chronic lung ... Chest CT: • Ground-glass ... : • Nephrotic syndrome ... (Epstein-Barr syndrome
Sjogren's Syndrome Overview

Epidemiology:
• F > M: 9:1
• 5-6th Decades (can be any age)

Autoimmune exocrinopathy multisystemic disease
salivary ducts Clinical ... Arthralgia and Arthritis ... MALT Lymphoma • Lung ... #Rheumatology # ... Diagnosis #Management
SAPHO Syndrome Summary

What?
SAPHO syndrome is a rare chronic inflammatory disorder of bone, joint, and skin characterized
nonerosive inflammatory arthritis ... and lower jaw Treatment ... osteoarticular and skin disease ... #Rheumatology # ... diagnosis #management
Polymyalgia Rheumatica (PMR) Diagnosis and Management Summary

WHAT?
 • Polymyalgia rheumatica (PMR) is a common systemic inflammatory
Diagnosis and Management ... CLINICAL: • Physical ... for giant cell arteritis ... TREATMENT: • The ... #Rheumatology
Pseudogout Summary - Calcium Pyrophosphate Deposition Disease (CPPD)

Pathophysiology:
Pyrophosphate produced by chondrocytes likely precipitates with calcium to
Acute Pseudogout: Treatment ... ≥2 attacks • Long-term ... Crowned Dens Syndrome ... #Rheumatology # ... diagnosis #management